Goat Anti-Wiskott-Aldrich Syndrome / WASP Antibody | LBP83194 | Leading Biology

商品分类：多克隆抗体

产品概述：The Wiskott-Aldrich syndrome (WAS) family of proteins share similar domain structure, and are involved in transduction of signals from receptors on the cell surface to the actin cytoskeleton. The presence of a number of different motifs suggests that they are regulated by a number of different stimuli, and interact with multiple proteins. Recent studies have demonstrated that these proteins, directly or indirectly, associate with the small GTPase, Cdc42, known to regulate formation of actin filaments, and the cytoskeletal organizing complex, Arp2/3. Wiskott-Aldrich syndrome is a rare, inherited, X-linked, recessive disease characterized by immune dysregulation and microthrombocytopenia, and is caused by mutations in the WAS gene. The WAS gene product is a cytoplasmic protein, expressed exclusively in hematopoietic cells, which show signalling and cytoskeletal abnormalities in WAS patients. A transcript variant arising as a result of alternative promoter usage, and containing a different 5' UTR sequence, has been described, however, its full-length nature is not known.

来源：Goat

存储溶液：0.5 mg IgG/ml in Tris saline (20mM Tris pH7.3, 150mM NaCl), 0.02% sodium azide, with 0.5% bovine serum albumin

参考文献：A congenital activating mutant of WASp causes altered plasma membrane topography and adhesion under flow in lymphocytes. Burns SO, et al. Blood, 2010 Jul 1. PMID 20354175.Customised molecular diagnosis of primary immune deficiency disorders in New Zealand: an efficient strategy for a small developed country. Ameratunga R, et al. N Z Med J, 2009 Oct 9. PMID 19859091.Characterization of Wiskott-Aldrich syndrome (WAS) mutants using Saccharomyces cerevisiae. Rajmohan R, et al. FEMS Yeast Res, 2009 Dec. PMID 19817875.Regulation of podosome dynamics by WASp phosphorylation: implication in matrix degradation and chemotaxis in macrophages. Dovas A, et al. J Cell Sci, 2009 Nov 1. PMID 19808890.The mechanism of CSF-1-induced Wiskott-Aldrich syndrome protein activation in vivo: a role for phosphatidylinositol 3-kinase and Cdc42. Cammer M, et al. J Biol Chem, 2009 Aug 28. PMID 19561083.

特异性：Human

别名：Wiskott-Aldrich syndrome protein, WASp, WAS, IMD2

简单描述：Goat Polyclonal Goat Anti-Wiskott-Aldrich Syndrome / WASP Antibody. Validated for use in WB, E.

详情：FunctionEffector protein for Rho-type GTPases. Regulates actin filament reorganization via its interaction with the Arp2/3 complex. Important for efficient actin polymerization. Possible regulator of lymphocyte and platelet function. Mediates actin filament reorganization and the formation of actin pedestals upon infection by pathogenic bacteria.Cellular_LocationCytoplasm, cytoskeleton.Tissue_LocationExpressed predominantly in the thymus. Also found, to a much lesser extent, in the spleen

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企业 QQ：3212874516、3198592576

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